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Paediatrics > Malignant Disease > Flashcards

Malignant Disease Flashcards

1
Q

Most common childhood cancers

A

Leukaemia followed by brain and spinal tumours

Then lymphomas

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2
Q

Ages incidence of leukaemia

A

Affects all ages but there is an early childhood peak

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3
Q

When are neuroblastoma and Wilms tumour seen

A

Almost always seen before 6 years old

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4
Q

When are Hodgkin lymphoma and bone tumours most seen?

A

Peak incidence in adolescence and early adult life

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5
Q

5 year survival of all children with cancers

A

75%

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6
Q

Which syndromes are associated with an increased risk of cancer?

A

Down syndrome associate with leukaemia

Neurofibromatosis associated with glioma

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7
Q

What can be used to identify bone marrow disease?

A

Nuclear medicine imaging eg. Radio labelled technetium bone scan

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8
Q

What is a tumour marker for neuroblastoma?

A

Urinary catecholamine excretion

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9
Q

What is tumour marker of germ cell tumours and liver tumours?

A

High alphafetoprotein production

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10
Q

Which age of childhood cancer has poorest prognosis?

A

Teenagers and young adults have poorer outcomes than children

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11
Q

Role of radiotherapy in childhood cancers

A

Retains a role in treatment of some tumours but the risk of damage to growth and function of normal tissues is higher than in adults
Therefore need to protect organs and tissues
Also keeping a child still is more difficult

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12
Q

When can bone marrow be useful in childhood cancers and when are the various types used?

A 
Allogenic transplants (from a compatible donor) of stem cells is principally used for high risk or relapsed leukaemia 
Autologous (from patient harvested beforehand) are most commonly used for children whose prognosis is poor when use high dose chemotherapy
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13
Q

What is the big risk in children with cancer?

A

Risk of serious infection due to being immunocompromised as a result of chemo and also the cancer
Therefore need to monitor for fever and neutropenia - if either then antibiotic treatment started

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14
Q

Which opportunistic infections are associated with cancer therapy? x3

A

PCP (especially in leukaemia patients) and disseminated fungal infections (aspergillosis and candidiasis)
Also coagulate-negative staph infections from CVC’s

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15
Q

Which viral infections are worse in cancer patient than a healthy child? x2

A

Chickenpox and measles can be life threatening
Other viral infections are no worse
Immunoglobulin for at risk children

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16
Q

GIT problems in children with cancer x3

A

Mouth ulcers are common therefore feeding is painful
Also chemotherapy agents are nauseating and induce vomiting
Therefore often do not feed well
Also chemotherapy induced but mucosal damage cause diarrhoea and may predispose to gram negative infection

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17
Q

What can doxorubicin cause?

A

Cardio toxicity

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18
Q

What can cyclophosphamide cause?

A

Haemorrhagic cystitis

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19
Q

What can cisplatin cause?

A

Renal failure and deafness

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20
Q

What can vincristine cause?

A

Neuropathy

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21
Q

Which is the most common leukaemia in children

A

Acute lymphoblastic leukaemia - accounts for 80% of all leukaemias in children

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22
Q

Peak age of presentation of ALL

A

2-5 years

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23
Q

How does ALL present? x10

A

Clinical symptoms and signs are from disseminated disease and systemic ill health from infiltration of bone marrow or other organs
Therefore malaise, anorexia, anaemia (pallor and lethargy), neutropenia (infections), thrombocytopenia (bruising, petechiae, nose bleeds), bone pain from infiltration
Hepatosplenomegaly or lymphadenopathy
CNS signs
Testicular enlargement

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24
Q

Onset of ALL normally

A

Normal presents insidiously over several weeks - but can progress very quickly

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25
Q

What will FBC show in ALL

A

Abnormal in most children, low hb, thrombocytopenia, evidence of circulating leukaemic blast cells

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26
Q

What investigation needs to be done to confirm ALL?

A

Bone marrow examination - essential to identify it but also to provide prognostic information

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27
Q

What other examination should be done in ALL?

A

Chest X-ray to look for mediastinal mass - characteristic of T cell disease

28
Q

What needs to be done before ALL treatment can begin? X4

A 
Correct anaemia (blood transfusion), decrease risk of bleeding by giving platelets, treat any infection 
Allopurinol (or urate oxidase) when risk is high and high white cell count - to protect renal function against effects of rapid cell lysis
29
Q

Treatment of ALL

A

4 weeks of combined chemotherapy - vincristine, steroid, methotrexate intrathecally (normal chemo doesn’t cross BBB)
Followed by 3 weeks consolidation (first 3 + thiopurine)
Then monthly maintenance vincristine + steroids, daily 6mcp and weekly methotrexate orally - for up to 3 years
Also delayed intensification at 16weeks to consolidate remission - lots of drugs

30
Q

What else is given to ALL patients

A

Co-trimoxazole prophylaxis against PCP

31
Q

Maintenance of remission with current treatment in ALL

A

Remission in 95%

32
Q

What sort of brain tumours do children get?

A

Almost always primary and 60% infratentorial (cerebellum)

33
Q

Most common child brain tumours x2

A

Astrocytoma (40%) - varies from benign to highly malignant

Medulloblastoma (20%) midline of posterior fossa and 20% have spinal mets at diagnosis

34
Q

Presentation of brain tumour in child

A

Often due to raised ICP but can be focal neurological signs

Detected depending on site

35
Q

Management of brain tumour x3

A

MRI
Surgery aiming at treating hydrocephalus and providing tissue diagnosis (biopsy) and doing maximum resection
(if possible - eg. not in brain stem)
Radiotherapy and chemo vary with tumour type and age of patient

36
Q

Which lymphomas are most common when?

A

Hodgkin most common in adolescence

Non Hodgkin most common in childhood

37
Q

How does Hodgkin lymphoma present? x4

A

Mostly painless lymphadenopathy
Can cause airway obstruction
Often long history (months) and
the systemic symptoms eg. sweating, pruritus, weight loss and fever are uncommon

38
Q

Investigation of Hodgkin lymphoma x3

A

Lymph node biopsy, imaging of all nodal sites and bone marrow biopsy

39
Q

Management of Hodgkin lymphoma

A

Chemo with or without radio

PET scan used to monitor treatment response

40
Q

Prognosis of Hodgkin lymphoma

A

Overall 80% can be cured and even disseminated disease 60% can be cured

41
Q

Prognosis of non Hodgkin lymphoma

A

Survival rates over 80% for B and T cell disease

42
Q

What is a neuroblastoma?

A

Arise from neural crest tissue in adrenal medulla and sympathetic nervous system

43
Q

When is neuroblastoma most common?

A

Before age of 5

44
Q

How do most children with neuroblastoma present?

A

Most present with abdominal mass but primary tumour can lie anywhere along sympathetic chain (from neck to pelvis)
Can be large and complex mass (enveloping major blood vessels and lymph nodes)
Also get pallor, weight loss, hepatomegaly (met), bone pain or limp (mets)

45
Q

What do children with neuroblastoma over 2 usually present with

A

Clinical symptoms from metastatic disease

46
Q

Investigations in neuroblastoma

A

Clinical and radiological features
Also raised catecholamine secretion
Confirmatory biopsy

47
Q

Prognosis of neuroblastoma

A

In very young infants - can spontaneously regress

Majority of children over 1 have advanced disease and poor prognosis

48
Q

Treatment of neuroblastoma

A

Local primary - surgery
Mets with chemotherapy high dose and autologous bone marrow transplant
Relapse rate is high and cure rate low (30%) with metastatic disease

49
Q

What is Wilms Tumour?

A

Nephroblastoma - embryonical renal tissue tumour - commonest renal tumour of childhood

50
Q

When do you see Wilms tumour?

A

80% before age 5 and rarely after 10

51
Q

Presentation of Wilms

A

Large abdominal mass usually found incidentally in otherwise well child
Can also uncommonly have abdominal pain, anorexia, anaemia (haemorrhage into mass), haematuria, hypertension

52
Q

Treatment of Wilms

A

Initial chemo and then nephrectomy - tumour then staged histologically and treatment decided on - radio restricted to advanced disease

53
Q

Prognosis for Wilms

A

Good with over 80% cure rate

If present with mets 60% cure rate

54
Q

Most common soft tissue sarcoma in children

A

Rhabdomyosarcoma (develop from skeletal muscle cells)

Wide variety of sites therefore varying presentation and prognosis

55
Q

Most common sites for rhabdomyosarcoma

A

Head and neck - 40% causing local signs

56
Q

When are bone tumours found

A

Uncommon before puberty

Osteogenic sarcoma more common than Ewing but Ewing more in younger children

57
Q

Gender predominance of bone tumours

A

Male predominance

58
Q

Common sites for bone tumours

A

Limbs most common site and bone pain most common feature - otherwise well

59
Q

Investigation of bone tumour x4

A

Plain X-ray for detection followed by MRI and bone scan

Chest CT to look for lung mets

60
Q

Treatment of bone tumours

A

Chemo before surgery - avoid amputation if possible - endoprosthetic resection of bone and replacement

61
Q

Presentation of retinoblastoma

A

Most within first 3 years of life with white pupillary reflex (not red) or with squint

62
Q

Treatment of retinoblastoma x3

A

Chemo and then laser therapy to retina

Radio in advanced disease

63
Q

Incidence of cancer before age of 15

A

1 in 500

64
Q

Inheritance of retinoblastoma

A

All bilateral are hereditary and about 20% of unilateral
Dominant inheritance but with incomplete penetrance
Susceptibility gene is on chromosome 13

65
Q

Prognosis of retinoblastoma

A

Most are cured but many are visually impaired

Paediatrics (16 decks)

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